Paraneoplastic neurological disorders in children with benign ovarian tumors

Hsu, Mei Hsin; Huang, Chao; Hung, Po-Cheng; Huang, Hsin‐Chun; Huang, Li; Huang, Chao; Sheen, Jiunn‐Ming; Huang, Song Chei; Chang, Ying

doi:10.1016/j.braindev.2013.04.009

Cited by 17 publications

(8 citation statements)

References 24 publications

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“…This conundrum is not limited to NB and OMS. An apparent duality of causation also upends a uniform model of autoimmune encephalitis, in which ovarian teratoma—rare in OMS—is found in a percentage of females that increases with age: 9% in those < 14 years old, 30% in those < 18 years old, and 56% of those > 18 years old . In contrast, teratoma‐associated N ‐methyl‐D‐aspartate receptor (NMDAR) encephalitis has not been reported in young males, and testicular teratoma is rare in men.…”

Section: Discussionmentioning

confidence: 99%

Multifactorial analysis of opsoclonus‐myoclonus syndrome etiology (“Tumor” vs. “No tumor”) in a cohort of 356 US children

Pranzatelli

Tate

McGee

2018

Pediatric Blood & Cancer

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Background: Pediatric opsoclonus-myoclonus syndrome (OMS) presents a paradox of etiopathogenesis: A neuroblastic tumor (NB) is found in only one half of the cases, the others are ascribed to infections or designated as idiopathic. Method:From an IRB-approved observational study of 356 US children with OMS, secondary analysis of "etiology" and related factors was performed on a well-characterized cohort. The "Tumor" (n = 173) and "No Tumor" groups (n = 183), as defined radiologically, were compared according to multiple factors considered potentially differentiating. Data were analyzed retrospectively using parametric and nonparametric tests as indicated.Results: Patients with NB were not distinguishable by prodromal symptoms, OMS onset age, gender, race/ethnicity, OMS severity, rank order of neurological sign appearance, or geographic distribution. Various CSF immunologic biomarker abnormalities of OMS did not vary in the presence or absence of a detectable tumor: frequency of six lymphocyte subsets, or concentrations of 18 cytokines/chemokines, cytokine antagonists, chemokine receptors, cell adhesion molecules, or neuronal/glial markers. Prior responsiveness to conventional immunotherapy was not contingent on tumor/no tumor designation. Conclusions:Multiple convergent factors provide compelling empirical evidence and rationalize the concept that OMS is one neurological disorder, regardless of apparent etiology. Limitations to the current clinical etiologic classifications as paraneoplastic, parainfectious/post-infectious, and idiopathic etiology require antigen-based biological solutions to tease out the molecular pathophysiology of viral/tumoral mechanisms. Systematic studies, regardless of presumed etiology, will be necessary to find the highest-yield combination of imaging approaches, screening for infectious agents, and new biomarkers. Two testable hypotheses for future research are presented.

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Section: Discussionmentioning

confidence: 99%

Multifactorial analysis of opsoclonus‐myoclonus syndrome etiology (“Tumor” vs. “No tumor”) in a cohort of 356 US children

Pranzatelli

Tate

McGee

2018

Pediatric Blood & Cancer

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“…Ovarian tumors are more common in female patients aged >10 years with an incidence of 58%, 31%, and 15%, for patients aged >18, <18, and <14 years, respectively, but is less common in patients aged <10 and in male patients. [ 3 , 5 , 33 ] Although the exact mechanism remains unknown, we speculate that the hormonal effect in reproductive females secondary to the estrogenic signaling in the development of ovarian teratoma might have some correlation with it. [ 34 ] With the possible hormonal effects, in anti-NMDAR encephalitis, paraneoplastic tumor seemed to be more prominent in the reproductive-female age groups, and became less observed in the nonreproductive groups and male patients.…”

Section: Discussionmentioning

confidence: 99%

“…[ 1 , 2 ] It predominantly affects young women with or without ovarian teratoma. [ 3 ] The clinical manifestations of anti-NMDAR encephalitis have a multistage course [ 4 , 5 ] : the prodromal phase includes nonspecific symptoms of headache, fever, vomiting, and general malaise, followed by a neuropsychiatric phase presenting with unusual manifestations, agitation, delirium, or acute psychotic changes. If the disease persists without proper diagnosis or treatment, the patients can deteriorate rapidly with language impairment; memory or cognitive impairment; and decreased responsiveness accompanied by hyper or hypokinetic movement disorders such as catatonia, dystonia, and chorea.…”

Section: Introductionmentioning

confidence: 99%

Variations of movement disorders in anti-N-methyl-D-aspartate receptor encephalitis

et al. 2016

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Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is one of the most common autoimmune encephalitis that presents with a wide variety of movement disorders. The purpose of our study is to review the manifestations and duration of movement disorders in different ages with NMDAR encephalitis.A retrospective cohort of 28 patients (20 females and 8 males) with positive cerebrospinal fluid (CSF) anti-NMDAR antibody in a 5-year period from major hospitals in Taiwan was enrolled. They were categorized into 3 age groups: 7 patients were ≤10 years, 14 patients were 10 to 18 years, and 7 patients were >18 years.Total 28 patients (20 females and 8 males) with age ranging from 8 months to 38 years were enrolled. Nearly all patients (n = 27/28, 96%) presented with at least 2 types of disorders, including orofacial–lingual dyskinesia (OFLD; n = 20), catatonia (n = 19), tremor (n = 11), bradykinesia (n = 11), dystonia (n = 11), choreoathethosis (n = 9), and ballism (n = 3). Only 1 patient below 10 years presented with isolated periodic choreoathethosis without other movement disorders. OFLD was common in all age groups. Choreoathetosis was most common in patients aged ≤10 years, while catatonia was most common in patients aged >10 years (P = 0.001 and 0.020, respectively). Bradykinesia was also more common in patients aged >10 years (P = 0.020). The clinical presentations of movement disorders were not significantly different in the age of 10 to 18 years and those >18 years. Neither patient ≤10 years old nor male patients had associated tumors. All patients’ movement disorders were improved after treatment, while female patients with tumors had worse short-term outcome (P = 0.014). Compared with other disorders, choreoathetosis persisted significantly longer in patients ≤10 years (P = 0.038), while OFLD and catatonia last longer in patients >10 years (P = 0.047 and 0.002, respectively).Our study shows that hyperkinetic movements such as choreoathetosis are more common and last longer in younger age groups, whereas hypokinetic movements such as catatonia and bradykinesia are more common and last longer in older age groups. Female patients with ovarian tumors had worse short-term outcome.

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“…[2][3][4] Similar paraneoplastic manifestations were noted in three patients with dysgerminomas. [6][7][8] Individual cases of paraneoplastic neurologic syndromes have also been reported in patients with teratomas [16][17][18] as well as hypercalcemia mediated by parathyroid-related protein 19 and a case of Cushing syndrome resulting from ectopic adrenocorticotropic hormone secretion caused by primary ovarian mature teratoma with carcinoid components. 20 Jaundice has only been previously reported in patients with ovarian carcinoma with metastatic disease to the hepatic hilar lymph nodes.…”

Section: Discussionmentioning

confidence: 94%